目的:我们旨在确定中国南方MillerFisher综合征(MFS)的临床特征,并与其他国家的临床特征进行比较。
方法:我们收集了2013-2016年诊断为MFS的患者的病历。我们分析了年龄,性别,发病季节,前兆事件,临床症状和体征,神经传导研究(NCS)的发现,脑脊液(CSF),治疗性补救措施,最低点时间,中国南方MFS患者的住院时间。我们同时比较了城市和农村地区以及不完全眼肌麻痹(IO)和完全眼肌麻痹(CO)患者之间的差异。
结果:该研究招募了72名患者:36名来自农村地区,36名来自城市地区,男50男22女.发病的平均年龄为47.72岁,30例(41.7%)和21例(29.2%)患者在春季和冬季发生MFS,分别。典型的眼肌麻痹三联征,共济失调,50例(69.4%)患者出现反射反应。52.8%的患者在发病前1周有上呼吸道感染史,而在CSF研究中,5.6%的人经历了胃肠道感染,48人(73.8%)出现了白蛋白细胞解离。只有26例(36.1%)患者出现NCS异常。此外,在经典MFS和急性眼肌麻痹的患者中,有83.5%的患者存在眼球向外运动受限,双侧对称性眼肌麻痹占64.2%。除了城市地区NCS异常的比例较高(47.2%与25.0%),性别比的城乡差异不大,发病年龄,高发季节,前兆事件,疾病特征,和脑脊液中的白蛋白细胞学解离。此外,CO患者年龄大于IO患者(64.53±7.69vs.43.19±14.40年[平均值±标准偏差],p<0.001)。
结论:MFS患者多为男性和中年人,大多数出现在冬天和(尤其是)春天。超过一半的患者有明显的前兆事件,其中大多数是典型的MFS与典型的三合会。超过70%的患者在CSF中出现白蛋白细胞解离。NCS异常在MFS中并不常见。IO患者的发病年龄低于CO患者;双侧对称性眼外肌麻痹是最常见的症状,外直肌是最常累及的肌肉。
OBJECTIVE: We aimed to determine the clinical features of Miller Fisher syndrome (MFS) in southern
China and compare them with those presenting in other countries.
METHODS: We collected the medical records of patients diagnosed with MFS during 2013-2016. We analyzed the age, sex, onset season, precursor events, clinical symptoms and signs, findings of nerve conduction studies (NCS), cerebrospinal fluid (CSF), therapeutic remedies, nadir time, and length of hospital stay of patients with MFS in southern
China. We concurrently compared the differences between urban and rural areas and between patients with incomplete ophthalmoplegia (IO) and complete ophthalmoplegia (CO).
RESULTS: The study enrolled 72 patients: 36 from rural areas and 36 from urban areas, and 50 males and 22 females. The mean age at onset was 47.72 years, and 30 (41.7%) and 21 (29.2%) patients developed MFS in spring and winter, respectively. The typical triad of ophthalmoplegia, ataxia, and areflexia was observed in 50 (69.4%) patients. A history of upper respiratory tract infection 1 week before onset was found in 52.8% of the patients, while 5.6% experienced gastrointestinal infections and 48 (73.8%) exhibited albuminocytological dissociation in the CSF study. Only 26 (36.1%) patients presented abnormalities in NCS. Moreover, restricted outward eyeball movement presented in 83.5% of the patients with classic MFS and acute ophthalmoplegia, and bilateral symmetrical ophthalmoplegia presented in 64.2%. With the exception of the higher proportion of NCS abnormalities in urban areas (47.2% vs. 25.0%), urban and rural differences were insignificant regarding sex ratio, age at onset, high-incidence season, precursor events, disease characteristics, and albuminocytological dissociation in the CSF. Furthermore, patients with CO were older than those with IO (64.53±7.69 vs. 43.19±14.40 years [mean±standard deviation], p<0.001).
CONCLUSIONS: The patients with MFS were mostly male and middle-aged, and most presented in winter and (especially) spring. More than half of the patients had clear precursor events, most of which were classic MFS with the typical triad. More than 70% of the patients presented albuminocytological dissociation in the CSF. NCS abnormalities were uncommon in MFS. The age at onset was lower in patients with IO than in patients with CO; bilateral symmetrical extraocular muscle paralysis was the most common symptom, and the external rectus was the most frequently involved muscle.